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Long-Term Efficacy and Safety of Multi-Matrix Mesalazine Maintenance Therapy in Pediatric Patients with Ulcerative Colitis. 多基质美沙拉嗪维持治疗小儿溃疡性结肠炎的长期疗效和安全性。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.44
Toshiaki Shimizu, Daisuke Tokuhara, Shigeo Nishimata, Takashi Ishige, Takahiro Kudo, Masahiro Takatsu, Kanako Nishimura, Akiko Matsuda, Koichi Hayashi, Katsuhiro Arai

Purpose: This study aimed to evaluate the efficacy and safety of multi-matrix mesalazine in pediatric patients with ulcerative colitis (UC) in remission.

Methods: A multicenter, open-label, uncontrolled study was conducted in Japan, in which multi-matrix mesalazine 40 mg/kg was administered orally once daily for 48 weeks to 23 patients with UC in remission, aged <17 years. The primary endpoint was the nonoccurrence of rectal bleeding based on the Ulcerative Colitis Disease Activity Index (UC-DAI) score.

Results: The percentage of patients without rectal bleeding, based on the UC-DAI score in the full analysis set, was 73.9% (two-sided 95% confidence interval [CI]: 51.6%, 89.8%; n=17/23). The lower limit of the two-sided 95% CI exceeded the predetermined threshold of 50%, derived from placebo group data in previous clinical studies involving adult patients with UC. The incidence of adverse events (AEs) and study drug-related AEs was 87.0% (n=20/23) and 13.0% (n=3/23), respectively. No deaths were reported. None of the study drug-related AEs were severe or serious, nor did they lead to discontinuation of the study drug.

Conclusion: This study demonstrated the long-term efficacy of multi-matrix mesalazine in pediatric patients with UC in remission in Japan. No noteworthy safety concerns beyond those known to be associated with mesalazine were observed.

目的:本研究旨在评价多基质美沙拉嗪治疗溃疡性结肠炎(UC)缓解期患儿的疗效和安全性。方法:在日本进行了一项多中心、开放标签、非对照研究,对23例老年UC缓解期患者口服多基质美沙嗪40 mg/kg,每日1次,持续48周。结果:基于全分析集中UC- dai评分,无直肠出血的患者比例为73.9%(双侧95%可信区间[CI]: 51.6%, 89.8%; n=17/23)。双侧95% CI的下限超过了预先设定的阈值50%,该阈值来自先前涉及成年UC患者的临床研究中安慰剂组的数据。不良事件(ae)和研究药物相关ae的发生率分别为87.0% (n=20/23)和13.0% (n=3/23)。没有死亡报告。所有与研究药物相关的不良事件都不是严重的,也没有导致研究药物的停药。结论:本研究证实了多基质美塞拉嗪对日本缓解期儿科UC患者的长期疗效。除了已知与美沙拉嗪相关的安全问题外,没有观察到值得注意的安全问题。
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引用次数: 0
Prospective Evaluation of a Novel Scoring System for Managing Foreign Body Ingestion in Children. 一种新的儿童异物摄入管理评分系统的前瞻性评价。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.35
Ryosuke Miyamoto, Hideyuki Iwayama, Sae Takahashi, Hitoshi Honma, Yu Masuda, Yoshinori Ito, Akihisa Okumura

Purpose: This study aimed to evaluate a novel scoring system for the management of foreign body ingestion in children. A simple scoring system can greatly aid in clinical decision-making.

Methods: We developed a novel scoring system that considers several critical factors, including patient age, mood, and symptoms, as well as the characteristics of the ingested foreign body, such as its type, location, and size. This system was designed to help emergency department residents determine the most appropriate course of action. Based on their scores, the children were categorized into two classes: Class A (observation) and Class B (pediatrician consultation), and further subcategorized as Class B1 and B2.

Results: A total of 158 children were evaluated using this scoring system, with 79 confirmed cases of foreign body ingestion. Class A patients had a higher rate of successful observation and minimal intervention, whereas Class B patients had a higher rate of invasive treatment. Importantly, there were very few unnecessary invasive procedures, and the recommendations made by the scoring system were closely aligned with those made by a reference management algorithm.

Conclusion: Our novel scoring system demonstrates substantial potential for guiding residents in the appropriate management of pediatric foreign body ingestion, ensuring that children receive the most suitable level of care.

目的:本研究旨在评估一种新的儿童异物摄入管理评分系统。一个简单的评分系统可以极大地帮助临床决策。方法:我们开发了一种新的评分系统,该系统考虑了几个关键因素,包括患者的年龄、情绪和症状,以及摄入的异物的特征,如其类型、位置和大小。该系统旨在帮助急诊科的居民确定最合适的行动方案。根据患儿的得分分为A类(观察类)和B类(儿科医师会诊类),再细分为B1类和B2类。结果:158名儿童使用该评分系统进行评估,其中79例确诊为异物摄入。A类患者观察成功率高,干预少,B类患者侵入性治疗率高。重要的是,很少有不必要的侵入性程序,评分系统提出的建议与参考管理算法提出的建议密切一致。结论:我们的新评分系统在指导住院医师正确处理儿童异物摄入方面显示了巨大的潜力,确保儿童获得最合适的护理水平。
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引用次数: 0
Diagnosis of Pediatric Inflammatory Bowel Disease (PIBD)-Implications for the Asia-Pacific Region: A Position Paper from the Asian Pan-Pacific Society for Pediatric Gastroenterology, Hepatology, and Nutrition PIBD Working Group. 儿科炎症性肠病(PIBD)的诊断-对亚太地区的影响:亚太儿科胃肠病学、肝病学和营养学协会PIBD工作组的立场文件。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.22
Rohan Malik, Marion M Aw, Way Seah Lee, Andy Darma, Pornthep Tanpowpong, Nuthapong Ukarapol, James Guoxian Huang, Hong Ngoc Tran, Yen Hsuan Ni

A significant increase in the incidence of pediatric inflammatory bowel disease (PIBD) across the Asia Pacific region has been observed. Countries in the region have diverse economies, with both developed and resource limited economies being represented. Literature from the region also varies, from reports on detailed epidemiological data and nuanced therapeutic outcomes derived from PIBD registries to case series from several geographic areas that have previously not reported on the condition. Through this position paper, we aimed to provide a comprehensive review of the challenges faced with diagnosing PIBD in the Asia Pacific region and offer pragmatic recommendations based on region-specific literature.

在整个亚太地区,儿童炎症性肠病(PIBD)的发病率显著增加。本区域各国的经济各不相同,既有发达经济体,也有资源有限经济体。来自该地区的文献也各不相同,从来自PIBD登记处的详细流行病学数据和细致入微的治疗结果的报告到以前未报道过该疾病的几个地理区域的病例系列。通过这份立场文件,我们旨在全面回顾亚太地区诊断PIBD所面临的挑战,并根据特定地区的文献提供实用的建议。
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引用次数: 0
Etiologies of Cyclic Vomiting Syndrome in Children: A Systematic Review of 1,373 Patients. 儿童周期性呕吐综合征的病因:1373例患者的系统回顾。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.62
Agostina Waisfeld, Isabel Randazzo, Stephen Aronoff, Laurence Feinstein, Michael Del Vecchio

Purpose: Cyclic vomiting syndrome (CVS), is a diagnosis of exclusion within the disorders of the gut-brain interaction, consisting of recurrent vomiting episodes unexplained by another medical condition or its triggers. When a patient presents with a recurrent vomiting episode, a broad differential is considered. The aim of this systematic review is to provide a hierarchal differential diagnostic approach to children with known or possible recurrent vomiting.

Methods: This study followed the Preferred Reporting Items in Systematic Reviews and Meta-Analyses (PRISMA) guidelines. PubMed, EMBASE and Scopus databases were searched using the following criteria: (1) Subjects aged 18 or under with vomiting spells classified as cyclic vomiting syndrome according to Rome IV criteria or equivalents for older publications and; (2) experimental, observational, or cross-sectional studies of 10 or more patients. A comprehensive literature search was performed with specific search criteria that included the diagnosis of cyclic vomiting syndrome. The results were analyzed using a Bayesian methodology to determine the rate estimates and 95% credible intervals for each disease.

Results: Seventeen studies (1,375 patients and 1,415 events) were recovered. 80.4% of the differential included: psychological stress (26.2%; 95% credible intervals [CredI], 24-28.5), infection (22%; 95% CredI, 19.9-24.2), motion sickness (19.3%; 95% CredI, 17.3-21.4) and migraine (12.9%; 95% CredI, 11.2-14.7). Surgical causes (4.8%; 95% CredI, 3.6-6.2) and mitochondrial diseases (0.4%; 95% CredI, 0.1-0.8) were uncommon.

Conclusion: Many triggers and diagnoses are associated with recurrent vomiting and CVS: psychological stress, infection, motion sickness, and migraine accounted for most cases. Serious surgical or metabolic diseases were less common.

目的:周期性呕吐综合征(CVS),是一种排除在肠-脑相互作用疾病中的诊断,包括无法解释的其他医学条件或其触发因素的反复呕吐发作。当患者出现复发性呕吐时,应考虑广泛的鉴别。本系统综述的目的是为已知或可能复发性呕吐的儿童提供分级鉴别诊断方法。方法:本研究遵循系统评价和荟萃分析(PRISMA)指南中的首选报告项目。PubMed、EMBASE和Scopus数据库采用以下标准进行检索:(1)受试者年龄在18岁或以下,根据Rome IV标准或同等标准将呕吐症状归类为周期性呕吐综合征;(2) 10名或以上患者的实验、观察或横断面研究。我们进行了全面的文献检索,检索标准包括周期性呕吐综合征的诊断。使用贝叶斯方法对结果进行分析,以确定每种疾病的发生率估计值和95%可信区间。结果:17项研究(1375例患者,1415个事件)恢复。80.4%的差异包括:心理压力(26.2%;95%可信区间[CredI], 24-28.5),感染(22%;95%可信区间[credit i], 19.9-24.2),晕动病(19.3%;95%可信区间[credit i], 17.3-21.4)和偏头痛(12.9%;95%可信区间[credit i], 11.2-14.7)。手术原因(4.8%;95% credit i, 3.6-6.2)和线粒体疾病(0.4%;95% credit i, 0.1-0.8)不常见。结论:许多诱因和诊断与复发性呕吐和CVS相关:心理压力、感染、晕动病和偏头痛占多数。严重的外科或代谢性疾病较少见。
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引用次数: 0
Congenital Hepatic Fibrosis and/or Autosomal Recessive Polycystic Kidney Disease: A Single-center Experience. 先天性肝纤维化和/或常染色体隐性多囊肾病:单中心经验。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.55
Derya Altay, Sibel Yel, İsmail Dursun, Duran Arslan

Purpose: Congenital hepatic fibrosis (CHF) and/or autosomal recessive polycystic kidney disease (ARPKD) represent rare and complex clinical conditions in childhood. Diagnostic challenges often arise due to heterogeneity in clinical manifestations.

Methods: This study included pediatric patients diagnosed with CHF and/or ARPKD who were followed by the Pediatric Gastroenterology and Pediatric Nephrology Departments. Patient records were reviewed retrospectively.

Results: A total of 23 patients were included in the study. The median age of the cohort was 12.7±4.8 years, and the median age at diagnosis was 0.6±3.4 years. Thirteen patients had combined CHF and ARPKD, while 10 had isolated ARPKD. The diagnosis was incidental in 13 patients (56.5%), whereas five patients (21.7%) presented with an abdominal mass. Most patients had mutations in the polycystic kidney and hepatic disease 1 gene. Bilateral kidney enlargement and multiple millimetric cysts were identified in the majority of cases. Three patients required organ transplantation during follow-up. Two patients who underwent liver or kidney transplantation experienced no complications, whereas the patient who received combined liver and kidney transplantation developed kidney failure secondary to reflux nephropathy. Except for one patient who died in infancy, disease progression was generally mild in the cohort.

Conclusion: Although kidney involvement is often predominant, hepatic complications may develop over time, particularly in patients with combined disease. A collaborative, multidisciplinary approach is essential for effectively managing the complex manifestations of these ciliopathies.

目的:先天性肝纤维化(CHF)和/或常染色体隐性多囊肾病(ARPKD)是儿童罕见而复杂的临床疾病。由于临床表现的异质性,诊断常常出现困难。方法:本研究纳入了诊断为CHF和/或ARPKD的儿科患者,这些患者由儿科消化内科和儿科肾脏内科随访。回顾性回顾患者记录。结果:共纳入23例患者。队列的中位年龄为12.7±4.8岁,诊断时的中位年龄为0.6±3.4岁。合并CHF和ARPKD 13例,单独ARPKD 10例。13例(56.5%)患者的诊断是偶然的,而5例(21.7%)患者表现为腹部肿块。大多数患者有多囊肾和肝脏疾病1基因突变。双侧肾脏肿大和多发毫米囊肿在大多数病例中被发现。随访中有3例患者需要器官移植。2例接受肝或肾移植的患者没有出现并发症,而接受肝肾联合移植的患者出现继发于反流肾病的肾衰竭。除了一名患者在婴儿期死亡外,该队列中的疾病进展通常较轻。结论:虽然肾脏受累通常是主要的,但肝脏并发症可能随着时间的推移而发生,特别是在合并疾病的患者中。协作,多学科的方法是必不可少的有效管理这些纤毛病的复杂表现。
{"title":"Congenital Hepatic Fibrosis and/or Autosomal Recessive Polycystic Kidney Disease: A Single-center Experience.","authors":"Derya Altay, Sibel Yel, İsmail Dursun, Duran Arslan","doi":"10.5223/pghn.2026.29.1.55","DOIUrl":"10.5223/pghn.2026.29.1.55","url":null,"abstract":"<p><strong>Purpose: </strong>Congenital hepatic fibrosis (CHF) and/or autosomal recessive polycystic kidney disease (ARPKD) represent rare and complex clinical conditions in childhood. Diagnostic challenges often arise due to heterogeneity in clinical manifestations.</p><p><strong>Methods: </strong>This study included pediatric patients diagnosed with CHF and/or ARPKD who were followed by the Pediatric Gastroenterology and Pediatric Nephrology Departments. Patient records were reviewed retrospectively.</p><p><strong>Results: </strong>A total of 23 patients were included in the study. The median age of the cohort was 12.7±4.8 years, and the median age at diagnosis was 0.6±3.4 years. Thirteen patients had combined CHF and ARPKD, while 10 had isolated ARPKD. The diagnosis was incidental in 13 patients (56.5%), whereas five patients (21.7%) presented with an abdominal mass. Most patients had mutations in the <i>polycystic kidney and hepatic disease 1</i> gene. Bilateral kidney enlargement and multiple millimetric cysts were identified in the majority of cases. Three patients required organ transplantation during follow-up. Two patients who underwent liver or kidney transplantation experienced no complications, whereas the patient who received combined liver and kidney transplantation developed kidney failure secondary to reflux nephropathy. Except for one patient who died in infancy, disease progression was generally mild in the cohort.</p><p><strong>Conclusion: </strong>Although kidney involvement is often predominant, hepatic complications may develop over time, particularly in patients with combined disease. A collaborative, multidisciplinary approach is essential for effectively managing the complex manifestations of these ciliopathies.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":"29 1","pages":"55-61"},"PeriodicalIF":1.6,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12816366/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146018756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis. APSPGHAN关于内镜下静脉曲张出血止血的声明。
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.1
Nuthapong Ukarapol, Ujjal Poddar, Tak Yau Stephen Lui, Yeoun Joo Lee, Robert N Lopez, Sittichoke Prachuapthunyachart, Nguyen Viet Truong, Shuichiro Umetsu, Ajay Sharma, Yao-Jong Yang, Nagina Shahzadi, Ruey Terng Ng, Rohan Malik, Maribel Vitug-Sales, Katsuhiro Arai, Way Seah Lee, Kyung Mo Kim

Purpose: Endoscopic variceal hemostasis is a critical, life-saving intervention frequently performed by pediatric gastroenterologists. To ensure safe and effective practices, the Asian Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition Endoscopy Scientific Subcommittee developed a consensus-based position statement.

Methods: A comprehensive literature review was conducted with an emphasis on pediatric evidence. Nineteen draft statements were formulated and refined through electronic votes and virtual meetings. Statements with <80% agreement were revised until consensus was reached. Levels of evidence certainty were evaluated to support the final recommendations.

Results: Nineteen position statements, covering screening, acute management, and surgical options, were endorsed. Although noninvasive tools such as elastography and platelet-based indices may assist in risk stratification, endoscopy remains the diagnostic and therapeutic gold standard. Endoscopic variceal ligation was identified as the preferred modality for both prophylaxis and treatment in children ≥10 kg, outperforming sclerotherapy and non-selective beta-blockers. Standard acute management incorporates octreotide and restrictive transfusion strategies, with endoscopy performed within 24 hours. In refractory cases, the Meso-Rex stunt or transjugular intrahepatic portosystemic shunt may be considered depending on anatomy and available expertise.

Conclusion: These findings provide an evidence-based framework for the management of pediatric variceal bleeding. Individualized care is emphasized while highlighting the pressing need for high-quality pediatric studies to strengthen future recommendations.

目的:内镜下静脉曲张止血是儿科胃肠病学家经常进行的一项关键的挽救生命的干预措施。为了确保安全有效的操作,亚太儿科胃肠病学、肝病学和营养内窥镜科学小组委员会制定了一项基于共识的立场声明。方法:以儿科证据为重点,进行全面的文献综述。通过电子投票和虚拟会议拟订和完善了19份声明草案。结果声明:19个立场声明,涵盖筛查,急性管理和手术选择,被认可。尽管弹性成像和血小板指数等非侵入性工具可能有助于风险分层,但内窥镜检查仍然是诊断和治疗的金标准。内镜下静脉曲张结扎被确定为预防和治疗≥10kg儿童的首选方式,优于硬化疗法和非选择性β受体阻滞剂。标准的急性治疗包括奥曲肽和限制性输血策略,并在24小时内进行内窥镜检查。在难治性病例中,根据解剖结构和可用的专业知识,可以考虑Meso-Rex特技或经颈静脉肝内门静脉分流术。结论:这些发现为小儿静脉曲张出血的治疗提供了循证框架。个性化护理强调,同时强调迫切需要高质量的儿科研究,以加强未来的建议。
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引用次数: 0
Daily Sodium Intake Based on Random Urinary Excretion in Children Aged 6-24 Months and Associated Risk Factors. 基于6-24月龄儿童随机尿排泄的每日钠摄入量及相关危险因素
IF 1.6 Q3 PEDIATRICS Pub Date : 2026-01-01 Epub Date: 2026-01-06 DOI: 10.5223/pghn.2026.29.1.71
Ronald Rompies, Yoga Devaera, Aryono Hendarto, Bernie Medise, Setyo Handryastuti, Aidah Baso, Miske Marsogi, Pramita Gayatri

Purpose: Excessive sodium intake in children can increase the risk of noncommunicable diseases in adulthood. Globally, children below 5 years of age consume approximately 2,000 mg of sodium daily, exceeding the recommended limit. However, studies on sodium intake among Indonesian children, especially those below 2 years of age, are limited. The aim of this study was to evaluate daily sodium intake and its associated risk factors.

Methods: In this cross-sectional study, the daily sodium intake and its associated risk factors were assessed in 85 healthy children aged 6-24 months in Jakarta between June and August 2024. Sodium intake was measured using urinary excretion (International Study on Salt and Blood Pressure formula), and dietary analysis was conducted using the Food Frequency Questionnaire. High sodium intake was defined per the Indonesian Pediatric Society guidelines.

Results: Results showed that 64% of children aged 6-11 months consumed over 1,200 mg/day of sodium (mean: 1,289.20±639.25), while 37% of children aged 12-24 months had high sodium intake (mean: 1,242.03±584.35). Family socioeconomic status significantly correlated with high sodium intake (r=0.176). Specific food types were identified as risk factors for excessive sodium consumption. In this study, it was concluded that almost all children aged 6-24 months had sodium levels above the recommended levels. Notably, all children aged 6-11 months had a higher sodium intake than approximately one-third of children aged 12-24 months.

Conclusion: These findings highlight the need for targeted interventions to reduce sodium intake among young children, especially among families with higher socioeconomic status.

目的:儿童钠摄入量过高可增加成年后非传染性疾病的风险。在全球范围内,5岁以下儿童每天摄入约2,000毫克钠,超过了建议的限量。然而,关于印尼儿童,特别是2岁以下儿童钠摄入量的研究是有限的。这项研究的目的是评估每日钠摄入量及其相关的危险因素。方法:在这项横断面研究中,对2024年6月至8月期间雅加达85名6-24月龄健康儿童的每日钠摄入量及其相关危险因素进行了评估。通过尿排泄量测量钠摄入量(国际盐和血压研究公式),并使用食物频率问卷进行饮食分析。高钠摄入量是根据印尼儿科学会指南定义的。结果:6 ~ 11月龄儿童钠摄入量超过1200mg /d的占64%(平均:1298.20±639.25),12 ~ 24月龄儿童钠摄入量高的占37%(平均:12242.03±584.35)。家庭社会经济地位与高钠摄入量显著相关(r=0.176)。特定的食物类型被确定为过量钠摄入的危险因素。在这项研究中,得出的结论是,几乎所有6-24个月大的儿童的钠含量都高于建议水平。值得注意的是,所有6-11个月大的儿童的钠摄入量都高于大约三分之一的12-24个月大的儿童。结论:这些发现强调需要有针对性的干预措施来减少幼儿,特别是社会经济地位较高的家庭的钠摄入量。
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引用次数: 0
Dual Challenges: A Case Report of Hirschsprung's Disease and Anorectal Malformation in a Child. 双重挑战:儿童巨结肠病和肛肠畸形1例报告。
IF 1.6 Q3 PEDIATRICS Pub Date : 2025-11-01 Epub Date: 2025-11-06 DOI: 10.5223/pghn.2025.28.6.405
Yoshita Gupta, Leela Dhar Agrawal, Dinesh Kumar Upadhyay

Hirschsprung's disease (HD) and anorectal malformations (ARMs) are rare congenital conditions with an incidence of approximately 1 per 5,000 live births. While each condition presents unique challenges, their simultaneous occurrence is rare, complicating diagnosis and surgical management. A 6-year-old female with ARM, previously managed by colostomy and anorectoplasty, continued to experience stool passage issues. Further evaluation of colonic biopsies confirmed the diagnosis of aganglionosis and HD. A modified Duhamel procedure was performed to remove the aganglionic segment and restore bowel function. The postoperative care was uneventful, and the patient was discharged with normal bowel function. This case illustrates the diagnostic complexity of HD in patients with ARM in whom symptom overlap and diagnostic delays are common. Comprehensive evaluation and customized surgical management are essential along with long-term follow-up to monitor complications and improve quality of life.

巨结肠病(HD)和肛门直肠畸形(ARMs)是罕见的先天性疾病,发病率约为每5000例活产1例。虽然每种情况都有独特的挑战,但同时发生的情况很少,这使诊断和手术治疗变得复杂。一名6岁的女性ARM,先前通过结肠造口术和肛肠成形术治疗,继续经历大便通道问题。进一步的结肠活检证实了神经节病和HD的诊断。采用改良的Duhamel手术切除神经节段,恢复肠功能。术后护理顺利,患者出院时肠功能正常。该病例说明了在症状重叠和诊断延迟常见的ARM患者中诊断HD的复杂性。全面的评估和定制的手术管理是必要的,同时长期随访,以监测并发症和改善生活质量。
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引用次数: 0
Inflammatory Bowel Disease in Jordanian Children: A Tertiary Center Experience. 炎症性肠病在约旦儿童:三级中心经验。
IF 1.6 Q3 PEDIATRICS Pub Date : 2025-11-01 Epub Date: 2025-11-06 DOI: 10.5223/pghn.2025.28.6.393
Hala Almomani, Ayah Alsmadi, Nicole Issi, Maria AlShurman, Eyad Altamimi

Purpose: This study describes the clinical features, disease subtypes, laboratory findings, and outcomes of pediatric inflammatory bowel disease (IBD) in Jordan, with the aim of comparing regional and global trends.

Methods: We conducted a retrospective review of the medical records of patients diagnosed with IBD before the age of 18 years, who were followed up at the pediatric gastroenterology clinic at Jordan University Hospital between January 2015 and December 2022. The diagnoses were based on the revised Porto criteria.

Results: A total of 31 patients (61.3% male) were included in this study. The mean age at diagnosis was 8.7±4.75 years. Very early onset IBD (VEO-IBD) accounted for 38.7% of all cases. Crohn disease (51.6%) was more common than ulcerative colitis (UC) (29.0%) or unclassified IBD (19.4%). Diarrhea and abdominal pain were the most common symptoms. Steroids were used for remission induction in 90.3% of the cases, and %-aminosalicylic acid (5-ASA) was the most common maintenance therapy (48.4%). In CD patients, ileocolonic involvement (L3) was the most common (5, 29.4%), with non-stricturing, non-penetrating behavior (B1) in (10, 58.8%). Among UC patients, pancolitis was the predominant phenotype (3, 37.5%). C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) were elevated in (19, 61.3%) patients, while ESR was elevated in (16, 51.6%) patients, respectively. Anemia was observed in more than half of the patients (18, 58.1%). Steroid remission was steroids in (28, 90.3%) patients. 5-ASA was the most common maintenance therapy used in our cohort (15, 48.4%). Biological therapy was used to (8, 25.8%) patients.

Conclusion: Crohn's was more prevalent than UC in the pediatric cohort. Limited access to biologics and frequent treatment escalations highlight the need for improved therapeutic options and the development of a national IBD registry.

目的:本研究描述了约旦儿童炎症性肠病(IBD)的临床特征、疾病亚型、实验室结果和结局,目的是比较区域和全球趋势。方法:我们对2015年1月至2022年12月期间在约旦大学医院儿科胃肠病学诊所随访的18岁以下诊断为IBD的患者的病历进行回顾性分析。诊断基于修订后的波尔图标准。结果:本研究共纳入31例患者,其中男性61.3%。平均诊断年龄为8.7±4.75岁。极早发IBD (VEO-IBD)占所有病例的38.7%。克罗恩病(51.6%)比溃疡性结肠炎(29.0%)或未分类IBD(19.4%)更常见。腹泻和腹痛是最常见的症状。90.3%的病例使用类固醇诱导缓解,%-氨基水杨酸(5-ASA)是最常见的维持治疗(48.4%)。在CD患者中,最常见的是回肠结肠受累(L3)(5, 29.4%),非狭窄,非穿透行为(B1)(10, 58.8%)。UC患者以全结肠炎为主要表型(3.37.5%)。c反应蛋白(CRP)水平和红细胞沉降率(ESR)升高的患者分别为(19例,61.3%)和(16例,51.6%)。半数以上患者出现贫血(18.58.1%)。(28,90.3%)患者的类固醇缓解是类固醇。5-ASA是我们队列中最常用的维持治疗(15.48.4%)。采用生物治疗(8.25.8%)。结论:克罗恩病在儿科队列中比UC更为普遍。有限的生物制剂可及性和频繁的治疗升级突出了改进治疗方案和建立国家IBD登记处的必要性。
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引用次数: 0
Association Between Functional Constipation and Growth Status in Children Based on Anthropometric Indices. 基于人体测量指数的儿童功能性便秘与生长状况的关系。
IF 1.6 Q3 PEDIATRICS Pub Date : 2025-11-01 Epub Date: 2025-11-06 DOI: 10.5223/pghn.2025.28.6.352
Hanifah Oswari, Michelle Clarissa Junaidi, Jessica Sylvania Oswari, Regina Suriadi, Glen Lazarus, Muzal Kadim

Purpose: To evaluate the relationship between functional constipation (FC) and growth status in children using anthropometric indices (weight-for-height Z-score [WHZ] and height-for-age Z-score [HAZ]).

Methods: We conducted a retrospective cohort study at a pediatric primary care center in Jakarta, Indonesia, from December 2020 to December 2021. Medical records of 34 children with FC diagnosed using Rome IV criteria and 34 age- and sex-matched healthy controls were reviewed. Growth status was assessed using WHO WHZ and HAZ before and after polyethylene glycol treatment for at least six months. Data were analyzed using t-tests or nonparametric equivalents, with significance set at p<0.05.

Results: The mean age of our cohort was 3.17±2.11 years at baseline. For children with FC, the mean treatment duration was 17.15±7.22 months. Children with FC had lower WHZ at baseline than the control group (-0.21±0.73, compared to 0.62±0.85, p<0.001). Following treatment, those with FC had increased WHZ (0.18±1.19, p=0.028). No changes were observed in the HAZ before and after treatment (p>0.05).

Conclusion: FC in children is associated with the WHZ. The completion of FC treatment improved the WHZ in children compared with that at baseline. However, no association was identified between FC and HAZ before and after treatment completion.

目的:利用人体测量指标(身高体重Z-score [WHZ]和年龄身高Z-score [HAZ])评价儿童功能性便秘(FC)与生长状况的关系。方法:我们于2020年12月至2021年12月在印度尼西亚雅加达的一家儿科初级保健中心进行了一项回顾性队列研究。本文回顾了34例使用Rome IV标准诊断为FC的儿童和34例年龄和性别匹配的健康对照者的医疗记录。聚乙二醇处理前后至少6个月,采用WHO WHZ和HAZ评估生长状况。使用t检验或非参数等效检验对数据进行分析,结果具有显著性:我们的队列在基线时的平均年龄为3.17±2.11岁。FC患儿的平均治疗时间为17.15±7.22个月。FC患儿基线WHZ低于对照组(-0.21±0.73,对照组为0.62±0.85,pp=0.028)。治疗前后HAZ无明显变化(p < 0.05)。结论:儿童FC与WHZ有关。与基线相比,FC治疗的完成改善了儿童的WHZ。然而,在治疗完成前后,没有发现FC和HAZ之间的关联。
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Pediatric Gastroenterology, Hepatology & Nutrition
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